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Abstract Number: 1573

Early Scleroderma with Non-Raynaud’s Symptoms Prior to Raynaud’s Onset Is Associated with Rapid Progression to Diffuse Skin Disease and Joint Contractures

Iqtidar Hanif1, Shervin Assassi1, Maureen Mayes1, Meng Zhang1, Julio Charles1, John VanBuren2, Jessica Alvey2, Kimia Ghaffari2, Elana Bernstein3, Flavia Castelino4, Lorinda Chung5, Luke Evnin6, Tracy Frech7, Jessica Gordon8, Faye Hant9, Laura Hummers10, Dinesh Khanna11, Kimberly Lakin12, Dorota Lebiedz-Odrobina13, Yiming Luo14, Ashima Makol15, Jerry Molitor16, Duncan Moore17, Carrie Richardson18, Nora Sandorfi19, Ami Shah20, Ankoor Shah21, Victoria Shanmugam22, Virginia Steen23, Elizabeth Volkmann24, Carleigh Zahn11 and Brian Skaug1, 1UTHealth Houston Division of Rheumatology, Houston, TX, 2University of Utah, Salt Lake City, UT, 3Division of Rheumatology, Department of Medicine, Vagelos College of Physicians and Surgeons, Columbia University Irving Medical Center, New York, NY, 4Massachusetts General Hospital, Boston, MA, 5Stanford University, Woodside, CA, 6Scleroderma Research Foundation, Brisbane, CA, 7Vanderbilt University Medical Center, Nashville, TN, 8Division of Rheumatology, Weill Cornell Medical College, New York, NY, 9Medical University of South Carolina, Charleston, SC, 10Johns Hopkins University, Division of Rheumatology, Baltimore, MD, Ellicott City, MD, 11University of Michigan, Ann Arbor, MI, 12Hospital for Special Surgery, New York, NY, 13University of Utah, Cottonwood Heights, UT, 14Columbia University, New York, NY, 15Mayo Clinic, Rochester, MN, Rochester, MN, 16University of Minnesota, Minneapolis, MN, 17Northwestern Memorial Hospital, Chicago, IL, 18Northwestern University, Chicago, IL, 19University of Pennsylvania, Philadelphia, PA, 20Division of Rheumatology, Johns Hopkins University, Ellicott City, MD, 21Duke University, Durham, NC, 22Office of Autoimmune Disease Research, Office of Research on Women's Health, National Institutes of Health, Great Falls, VA, 23Georgetown University School of Medicine, Washington, DC, 24University of California, Department of Medicine, Los Angeles, CA, USA, Los Angeles

Meeting: ACR Convergence 2024

Keywords: Scleroderma, Systemic sclerosis

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Session Information

Date: Sunday, November 17, 2024

Title: Systemic Sclerosis & Related Disorders – Clinical Poster II

Session Type: Poster Session B

Session Time: 10:30AM-12:30PM

Background/Purpose: Raynaud’s phenomenon (RP) is often the initial clinical manifestation of systemic sclerosis (SSc).  RP alerts clinicians to the possibility of an autoimmune rheumatic disease, prompting examination for cutaneous and musculoskeletal signs and antinuclear antibodies (ANA).  However, some SSc patients develop other manifestations, particularly puffy hands, prior to the onset of RP, which can make early SSc more challenging to recognize.  In order to help elucidate the diversity of SSc presentation in its early stages, we describe the initial clinical manifestations and ANA profiles of patients whose initial SSc manifestation was not RP in two early SSc cohorts.

Methods: Two independent SSc cohorts were examined, both of which enrolled patients within five years of the first non-RP symptom.  Patients in cohort one (n = 450) met the 1980 ACR preliminary classification criteria or the 2013 ACR/EULAR classification criteria.  Patients in cohort two (n = 938) met the 2013 ACR/EULAR classification criteria.  The initial clinical manifestation of SSc, demographics, metrics of disease severity, and ANA results were determined and compared between those whose initial manifestation was RP vs. a non-RP symptom.

Results: 195/450 (43.3%), and 292/938 (31.1%) SSc patients in cohorts one and two, respectively, had a non-RP initial symptom (Table 1), the most common of which was puffy hands (Figure 1).  Non-RP first presentation was more common amongst Black patients compared to other race/ethnicity categories (Table 1).  Despite similar time from the first non-RP symptom to enrollment, non-RP first patients were more likely than RP first patients to have diffuse cutaneous involvement (71.8% vs. 52.9%, p < 0.001 in Cohort 1; 69.9% vs. 57.0%, p < 0.001 in Cohort 2), higher mean mRSS (19.3 vs. 14.1, p < 0.001 in Cohort 1; 15.1 vs. 11.1, p < 0.001 in Cohort 2), and joint contractures (66.0% vs. 52.8%, p = 0.013 in Cohort 1; 51.2% vs. 40.2%, p = 0.002 in Cohort 2) (Table 1).  In both cohorts, RNA Polymerase III antibody was more prevalent in non-RP first compared to RP first patients (26.3% vs. 14.9%, p = 0.003 in Cohort 1; 34.7% vs. 19.9%, p < 0.001 in Cohort 2) (Figure 2).  26/450 (5.8%) and 45/938 (4.8%) patients in cohorts one and two, respectively, had not experienced RP at all at the time of enrollment.  The majority of these patients had diffuse cutaneous involvement and joint contractures (data not shown).

Conclusion: In two independent cohorts, we found that >30% of SSc patients had begun to manifest SSc with puffy hands or other symptoms without the “warning sign” of RP as their initial symptom.  These patients tended to present with more severe skin and joint disease, highlighting the importance of early recognition.  The most common autoantibody associated with this presentation was RNA Polymerase III, which importantly is not included in some of the multiplex antibody panels often used as ANA screening tools in clinical practice.  These results should be considered in efforts to recognize SSc in its earliest stages.  Puffy hands, even in the absence of RP, should prompt consideration of early SSc and testing for ANA by an approach that is inclusive of RNA Polymerase III and other nucleolar antigens.

Supporting image 1

Table 1: Patient demographics and clinical characteristics by first SSc symptom status

Supporting image 2

Figure 1: Initial symptom in non-RP first patients

Supporting image 3

Figure 2: ANA/Autoantibody prevalence in non-RP versus RP first patients


Disclosures: I. Hanif: None; S. Assassi: AstraZeneca, 2, aTyr, 2, 5, BMS, 2, Boehringer-Ingelheim, 2, 5, CSL Behring, 2, Janssen, 5, Merck/MSD, 2, TeneoFour, 2; M. Mayes: AstraZeneca, 5, Atyr, 5, Boehringer-Ingelheim, 1, 5, Horizon Therapeutics, 5, Merck/MSD, 5; M. Zhang: None; J. Charles: None; J. VanBuren: None; J. Alvey: None; K. Ghaffari: None; E. Bernstein: AstraZeneca, 5, aTyr, 5, Boehringer-Ingelheim, 1, 2, 5, Bristol-Myers Squibb(BMS), 5, Cabaletta, 1, 5, Kadmon, 5; F. Castelino: Boehringer-Ingelheim, 1, Mediar Therapeutics, 1; L. Chung: Boehringer-Ingelheim, 5, Eicos, 1, 2, Eli Lilly, 2, Genentech, 2, IgM Biosciences, 2, Janssen, 1, Kyverna, 2, Mitsubishi Tanabe, 1, 2; L. Evnin: Eicos, 1, 11, Frontier Medicines, 4, 11, MPM Capital, 2, 8, Photys Therapeutics, 4, 11, Redona Therapeutics, 4, 11, Trishula Therapeutics, 4, 11, Umoja Biopharma, 4, 11, Werewolf Therapuetics, 4, 11; T. Frech: None; J. Gordon: None; F. Hant: None; L. Hummers: AbbVie/Abbott, 2, AstraZeneca, 5, Biotest, 1, 2, Boehringer-Ingelheim, 2, 5, Cumberland, 5, GlaxoSmithKlein(GSK), 5, Kadmon, 5, Medpace, 5, Merck/MSD, 5, Mitsubishi Tanabe, 5, prometheus, 5; D. Khanna: AbbVie/Abbott, 2, Amgen, 2, AstraZeneca, 2, Boehringer-Ingelheim, 2, Bristol-Myers Squibb(BMS), 2, Cabaletta, 2, Certa Therapeutics, 2, GlaxoSmithKlein(GSK), 2, Janssen, 2, MDI Therapeutics, 8, Merck/MSD, 2, Novartis, 2, Zura Bio, 2; K. Lakin: None; D. Lebiedz-Odrobina: None; Y. Luo: None; A. Makol: Amgen, 12, Site PI for clinical trial, AstraZeneca, 12, Site PI for Clinical trial, Boehringer-Ingelheim, 12, Site PI for Clinical Trial, Genentech, 12, Site PI for Clinical Trials; J. Molitor: None; D. Moore: None; C. Richardson: Cabaletta Bio, 2; N. Sandorfi: Bristol-Myers Squibb(BMS), 2, Novartis, 1; A. Shah: Arena Pharmaceuticals, 5, Kadmon, 5, Medpace LLC, 5; A. Shah: Amgen, 5, Boehringer-Ingelheim, 5; V. Shanmugam: None; V. Steen: None; E. Volkmann: AbbVie, 2, Boehringer-Ingelheim, 2, 5, 6, GSK, 2, 5, Horizon, 5, Kadmon, 5, Prometheus, 5; C. Zahn: Amgen, 12, Clinical Trial Investigator, Boehringer-Ingelheim, 12, Clinical Trials Investigator; B. Skaug: None.

To cite this abstract in AMA style:

Hanif I, Assassi S, Mayes M, Zhang M, Charles J, VanBuren J, Alvey J, Ghaffari K, Bernstein E, Castelino F, Chung L, Evnin L, Frech T, Gordon J, Hant F, Hummers L, Khanna D, Lakin K, Lebiedz-Odrobina D, Luo Y, Makol A, Molitor J, Moore D, Richardson C, Sandorfi N, Shah A, Shah A, Shanmugam V, Steen V, Volkmann E, Zahn C, Skaug B. Early Scleroderma with Non-Raynaud’s Symptoms Prior to Raynaud’s Onset Is Associated with Rapid Progression to Diffuse Skin Disease and Joint Contractures [abstract]. Arthritis Rheumatol. 2024; 76 (suppl 9). https://acrabstracts.org/abstract/early-scleroderma-with-non-raynauds-symptoms-prior-to-raynauds-onset-is-associated-with-rapid-progression-to-diffuse-skin-disease-and-joint-contractures/. Accessed .
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